Gonadoblastomas are unusual benign neoplasias that frequently appear in the dysgenetic gonads of women with chromosome Y anomaly. In this study, we reviewed 3 gonadoblastoma cases, 2 of which were bilateral, in patients 21, 17, and 18 years of age. Two of them presented 46 XY karyotype and gonadal dysgenesis, whereas the third presented 46 XX karyotype. Besides, 2 of the cases were associated to dysgerminomas. In all the cases, the histologic examination showed germ cell proliferation and sex cords derivatives frequently surrounding small round deposits containing amorphous hyaline material resembling Call-Exner bodies. One of the patients died at 8 years from diagnosis because of dysgerminoma multiple metastases, one is alive with no evidence of disease at the second year of follow-up, and the evolution of the third patient remains unknown.
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